Review Da Costa's Syndrome Or Neurocirculatory Asthenia
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Br Heart J 1987;58:306-15 Review Da Costa's syndrome or neurocirculatory asthenia OGLESBY PAUL From the Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA SUMMARY The syndrome variously called Da Costa's syndrome, effort syndrome, neuro- circulatory asthenia, etc has been studied for more than 100 years by many distinguished physi- cians. Originally identified in men in wartime, it has been widely recognised as a common chronic condition in both sexes in civilian life. Although the symptoms may seem to appear after infec- tions and various physical and psychological stresses, neurocirculatory asthenia is most often encountered as a familial disorder that is unrelated to these factors, although they may aggravate an existing tendency. Respiratory complaints (including breathlessness, with and without effort, and smothering sensations) are almost universal, and palpitation, chest discomfort, dizziness and faintness, and fatigue are common. The physical examination is normal. The aetiology is obscure but patients usually have a normal life span. Reassurance and measures to improve physical fitness are helpful. Da Costa's syndrome or neurocirculatory asthenia Although Jacob M Da Costa is the name most has a long and honourable history in the medical honoured by history in this condition, at least one literature and in clinical medicine. Yet it is infre- other relevant report antedated his paper in 1871. quently mentioned today. It is unlikely to have dis- On 3 June 1863 Dr Henry Hartshorne made a appeared; it probably exists much as before but is presentation regarding heart disease in the Union more often identified and labelled in psychiatric Army to the College of Physicians in Philadelphia. terms such as "anxiety state" or "anxiety neurosis". He commented: "Among the chronic affections of There is no harm in this shift in diagnostic labels as soldiers, which are best studied in hospitals remote long as the essential importance of the syndrome, its from the field, is one which does not seem to have prognosis, and treatment are properly appreciated. met, as yet, with full appreciation by medical Such is not always the case and, as in other medical officers, inspectors and pension surgeons... The issues, it is educational to review and summarise the affection to which I allude may be designated as mus- past. What has been forgotten should not necessarily cular exhaustion of the heart."' remain forgotten. Dr Hartshome went on to mention that Dr Alfred For the purposes of this discussion, a broad Stille had delivered an address on a somewhat simi- definition of Da Costa's syndrome that is applicable lar disorder, referred to by him as "palpitation", to military and civilian patients is: a disorder of before the Philadelphia County Medical Society unknown origin, often familial, characterised by the four months earlier.2 Stille had noted that this pal- presence of one or more symptoms including pitation was "a very frequent symptom among the breathlessness with and without effort, palpitation, soldiers, occurring in perhaps every case of inter- nervousness, chest discomfort not typical of angina costal neuralgia, but often, also, originating appar- pectoris caused by ischaemic heart disease, ently in a state of extreme exhaustion..." fatigability, and faintness; tending to occur in Hartshorne described how in an 80 bed ward of a attacks which may recur over years and for which Union army hospital over a seven month period the there is no specific treatment. majority of the cardiac patients exhibited neither hypertrophy nor dilatation, or palpitation "from sympathy with irritated stomach, from nervousness, Requests for reprints to Dr Oglesby Paul, Countway Library of abuse of tobacco, etc", but cardiac muscular exhaus- Medicine, Harvard Medical School, 10 Shattuck Street, Boston, tion. This was demonstrated by shortness of breath MA 02115, USA. after moderate exertion and a rapid pulse on slight Accepted for publication 19 May 1987 effort. The men appeared well and there were no 306 Da Costa's syndrome or neurocirculatory asthenia 307 cardiac murmurs; however, sometimes the first heart ady common among soldiers". He wrote a report sound was diminished. Although there was about it to the War Department as early as Decem- improvement with several months of rest this did ber 1862 and finally summarised his thoughts in the not cure most of the cases. He considered that the 36 page article published in 1871 in the American process was attributable to the stress of the military Journal of Medical Sciences.4 campaigns with "great and prolonged exertion with Da Costa's clear description of this malady was the most unfavorable conditions possible-privation supplemented by several case histories. Although of rest, deficient food, bad water, and malaria." the story he tells related only to soldiers, he made the Four year later in February 1867, Dr W C point in the first paragraph that "Much ofwhat I am Maclean, who was Professor of Military Medicine at about to say I could duplicate from the experience of the British Army Medical School, wrote a lecture private practice." Therefore, this was not a phenom- entitled "Diseases of the heart in the British Army", enon seen solely in military surroundings. Later in which was published in the British Medical Jour- the article he confirmed this by mentioning that nal.3 His message regarding British soldiers was some of his patients had experienced typical symp- different from that of Hartshorne because he was toms before enlisting. After commenting that he had calling attention to cardiac hypertrophy and chosen the name of "irritable heart" for this "pecu- dilatation caused, he considered, by excessive exer- liar form of functional disorder", which he believed tion from carrying the soldier's field pack, which had also occurred in British troops earlier in the cen- amounted to over 60 pounds, and by the manner in tury, he made the following observations. which its straps constricted the circulation. No The condition often followed a period of hard ser- symptoms are described in Maclean's paper and it vice in the field or a febrile illness with or without hardly belongs in this historical sequence started by diarrhoea; less frequently it was seen after various Hartshorne, although both were concerned with the situations including battle wounds and scurvy. The role of physical exhaustion. main symptoms were palpitation with a rapid pulse, The first major publication on the topic was enti- with and without effort, and on occasion with slight tled "On irritable heart: a clinical study of a form of irregularities; chest pain near the cardiac apex either functional cardiac disorder and its consequences" by sharp and lancinating, or dull in character, coming Dr Jacob M Da Costa, published in the January with and without effort; and shortness of breath, 1871 issue of the American Journal of Medical Sci- again not necessarily associated with exertion. A ences.4 Da Costa was only one of a series of notable range of digestive complaints was also common. The physicians to be intrigued by this problem, which physical examination was described as not remark- over the next 70 years included some of the great able except for the unusually rapid pulse, a quick names in internal medicine. cardiac impulse, and sometimes apical systolic mur- Da Costa was born in 1833 on the Island of St murs. Treatment was prolonged and included rest Thomas in the West Indies. He was educated in and digitalis preparations, at times combined with Europe and came to Philadelphia in 1849, where at aconite or other drugs. A few patients could return the age of 16 he entered Jefferson Medical College. to full duty when they had completely recovered, He graduated three years later at 19 and went on to but most had to be assigned light duty or were enrich his education with 18 months of observation retired from the service. Da Costa made the point in the clinics of Paris and Vienna before returning to that one infrequent outcome was the development of practise in Philadelphia. Wooley reviewed his dis- cardiac hypertrophy, which he believed to have tinguished career,5 which included publication in developed in 28 of 200 cases seen. Finally, he cau- 1864, when Da Costa was only aged 31, of a volume tioned that such a condition occurs with "severe or on medical diagnosis which was so successful that it protracted" military campaigns, and he urged ade- ran to nine editions and was translated into German, quate physical training for new recruits, avoidance Russian, and French. Da Costa was appointed to the of forced marches, and provision for adequate con- position of Chairman of Medicine at Jefferson Med- valescence for those with acute infections before ical College, was elected President of the College of they returned to duty. Physicians of Philadelphia, and was selected as one Such were the features of Da Costa's syndrome as of the original members and later president of the set out in 1871. It must be borne in mind that on the Association of American Physicians. one hand, some of the patients indeed did have or Da Costa's role in military medicine came during had had malaria, typhoid, acute gastroenteritis, the Civil War when Philadelphia became the site of scurvy, malnutrition, and physical exhaustion, and several military hospitals. Da Costa was assigned to had a reason for temporary asthenia, and Da Costa one at a suburban Philadelphia estate on Turner's considered these in his description. And on the other Lane. Here Da Costa was exposed to a "cardiac mal- hand, a few others may have had organic heart dis- 308 Paul ease that was undiagnosable at the time, including a 700 bed hospital at Colchester in Essex. Other coronary or hypertensive heart disease, myocarditis, smaller rehabilitation centres were opened in other and myocardiopathy.